Clinical ReviewAcquired ichthyosis
Section snippets
Clinical findings
Acquired ichthyosis (AI) most commonly manifests in adulthood and is otherwise difficult to distinguish from the hereditary forms of ichthyosis.3 Clinical manifestations include symmetric scaling, which ranges in severity from minor roughness and dryness to dramatic desquamation of platelike scales (Fig 1).4 The color of the scales varies from white to gray to brown, with a diameter ranging from less than 1 mm to greater than 1 cm.3 It primarily affects the trunk and limbs, typically being
Pathology
Histologically, both AI and ichthyosis vulgaris (IV) usually present with compact or laminated orthohyperkeratosis, a reduced or absent granular layer, and normal thickness of the spinous layer.1, 6 Absence of an inflammatory infiltrate in the dermis is also among the typical histologic findings.7, 8 Alternatively, epidermal atrophy and presence of a mild perivascular lymphohistiocytic infiltrate in the papillary dermis have also been observed.6
Pathogenesis
Ichthyosis occurs when there is a disruption in the process of cornification, resulting in hyperkeratosis, scaling, and abnormalities of the stratum corneum barrier function. A thickened stratum corneum is the result of cells entering this compartment at an increased rate or prolonged retention of corneocytes.
There are numerous ichthyosiform dermatoses, and these disorders all have in common abnormal cornification. IV, which is the most common inherited ichthyosis, is caused by mutations in
Diagnosis
The first step in evaluating an adolescent or adult with new-onset ichthyosis is differentiating AI from other causes of new-onset ichthyosis, such as late-onset IV, xerosis, and Refsum's disease. Essentially all other hereditary forms of ichthyosis will present well before age 13 years and, therefore, do not need to be considered in this setting.
IV is an autosomal dominant disease and, therefore, a positive family history is typical, unlike in AI. Patients develop dry, rough skin that usually
Association with systemic diseases
Once the diagnosis of AI is established, focus must change to evaluating the patient for underlying causes. AI may be a result of malignant disease, nonmalignant disease, or a drug reaction (Table I). If associated with a systemic disease, the cutaneous manifestations can present either before or after identification of the associated disease.5 The severity of AI may depend on the severity and acuteness of the internal disease.11 The ichthyosis typically regresses once the underlying disease
Medications
Cimetidine, a commonly used histamine blocker,69 has been reported to cause AI.51, 69, 70 It has been shown that in addition to its histamine-blocking effects, cimetidine is also a competitive inhibitor of dihydrotestosterone, and ichthyosis may be related to this antiandrogenic activity.6
Clofazimine, a riminophenazine derivative that is used in the management of leprosy,6, 71 has been linked to AI.6, 51, 71 Ichthyosis arises only when the dosage reaches 100 mg/d.71 In a study of 8 patients
Diagnostic considerations
Once the diagnosis of AI is established or strongly suggested, the initial focus must be identifying an underlying cause. It is important to remember that the skin manifestations may appear before or after the identification of underlying disease. The most important initial step in the evaluation is a detailed history, review of symptoms, and physical examination.
Specific questioning should inquire about medication use, risk factors for HIV, constitutional symptoms, history of liver or kidney
Treatment
For general treatment, hydration, lubrication, and keratolysis are all useful.10 In addition, the impaired barrier function of the skin associated with ichthyosis predisposes patients to skin infections.10 Because of this predisposition, consideration should be given to prophylactic measures, such antiseptic soaps containing triclosan or chlorhexidine. If skin infections develop, topical and systemic antibacterials should be quickly instituted.10
Hydration encourages desquamation by increasing
Conclusion
The management of AI is challenging and complex because of its association with numerous systemic diseases and drugs. We have reviewed the recent literature on this topic and have offered suggestions for the evaluation of these patients.
Because there are a large number of diseases, medications, and physiologic conditions reported in association with AI, the diagnostic evaluation must be conducted in a thorough, organized manner. In general, treatment should be directed at the underlying disease
References (77)
- et al.
The Refsum disease marker phytanic acid, a branched chain fatty acid, affects Ca2+ homeostatis and mitochondria, and reduces cell viability in rat hippocampal astrocytes
Neurobiol Dis
(2005) - et al.
Acquired ichthyosis, alopecia and loss of hair pigment associated with leiomyosarcoma
J Eur Acad Dermatol Venereol
(1998) - et al.
Mycosis fungoides presenting as an acquired ichthyosis
J Am Acad Dermatol
(1996) - et al.
Ichthyosiform mycosis fungoides: an atypical variant of cutaneous T-cell lymphoma
J Am Acad Dermatol
(2004) - et al.
Lymphomatoid papulosis associated with acquired ichthyosis
J Am Acad Dermatol
(1994) - et al.
Acquired ichthyosis: a paraneoplastic skin manifestation of Hodgkin's disease
Lancet Oncol
(2002) - et al.
Acquired ichthyosis in a patient with acquired immunodeficiency syndrome and Kaposi's sarcoma
J Am Acad Dermatol
(1987) - et al.
Acquired ichthyosis associated with dermatomyositis
J Am Acad Dermatol
(1987) - et al.
Acquired ichthyosis in bone marrow transplant recipients
J Am Acad Dermatol
(1996) - et al.
Ichthyosiform sarcoidosis: report of two cases and a review of the literature
J Am Acad Dermatol
(1987)
Acquired ichthyosiform erythroderma and sarcoidosis
J Am Acad Dermatol
The permeability barrier in essential fatty acid deficiency; evidence for a direct role for linoleic acid in barrier function
J Invest Dermatol
Prevalence of cutaneous disease in patients with acquired immunodeficiency syndrome (AIDS) or AIDS-related complex
J Am Acad Dermatol
Dermatologic findings related to human immunodeficiency virus infection in high-risk individuals
J Am Acad Dermatol
Papulosquamos dermatoses of AIDS
J Am Acad Dermatol
Acquired ichthyosis in concomitant HIV-1 and HTLV-II infection: a new association with intravenous drug abuse
J Am Acad Dermatol
Identity of HTLV-1 associated tropical spastic paraparesis and HTLV-1 associated myelopathy
Lancet
Disorders of keratinization
Dermatological writings of ancient India
Med Hist
Acquired ichthyosis associated with systemic lupus erythematosus
Lupus
Ichthyosis vulgaris: a clinical and histopathological study of patients and their close relatives in the autosomal dominant and sex-linked forms of the disease
Acta Derm Venereol Suppl (Stockh)
Hereditary and acquired ichthyosis vulgaris
Int J Dermatol
Acquired ichthyosis and related conditions
Int J Dermatol
Acquired ichthyosis–a sign of nonlymphoproliferative malignant disorders
Arch Dermatol
Ichthyosis etiology, diagnosis and management
Am J Clin Dermatol
Acquired ichthyosis: a marker for internal disease
Am Fam Physician
Ichthyosiform atrophy of the skin in Hodgkin's disease
Arch Dermatol Syph
Acquired ichthyosis in Hodgkin's disease
Br Med J
Acrokeratosis paraneoplastica (Bazex syndrome) occurring with acquired ichthyosis in Hodgkin's disease
Br J Dermatol
Acquired ichthyosis disclosing Hodgkin's disease: simultaneous recurrence
Presse Med
Acquired ichthyosis and impaired dermal lipogenesis in Hodgkin's disease
Br J Dermatol
Acquired ichthyosis: multiple causes for an acquired generalized disturbance in desquamation
Br J Dermatol
Anaplastic large-cell lymphoma associated with acquired ichthyosis
J Am Acad Dermatol
Acquired ichthyosis as a manifestation of abdominal recurrence of non-Hodgkin's lymphoma
Am J Hematol
Paraneoplastic acquired ichthyosis revealing non-Hodgkin's lymphoma
Ann Dermatol Venereol
Ichthyosis: early manifestation of intestinal leiomyosarcoma
Br Med J
Erythema gyratum repens and acquired ichthyosis associated with transitional cell carcinoma of the kidney
Clin Exp Dermatol
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2022, Anais Brasileiros de DermatologiaCitation Excerpt :Ichthyoses can be hereditary or acquired. The hereditary forms are usually present at birth and their onset may occur later until the age of 13.1 Acquired ichthyoses (AI) can be associated with different etiologies, such as malignant, infectious, autoimmune diseases, endocrinological and nutritional disorders, kidney and liver failure, drug reactions and sarcoidosis.
Hypothyroidism revealed by acquired ichthyosis in an adult patient
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2019, JAAD Case ReportsCitation Excerpt :Similar ichthyotic dermatoses have been reported in at least 3 other cases of PDCB ingestion, including 2 twin women and a middle age woman.1,2 PDCB has also been reported to cause neurologic symptoms including encephalopathy, tremors, and muscle weakness in patients with exposure for more than a year.3-7 As with our patient, most of the other reported patients had a history of preceding psychiatric illness.
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Conflicts of interest: None identified.